Lessons From Leprosy: Peripheral Neuropathies and Deformities in Chronic Demyelinating Diseases.

In light of the World Health Organization's push to accelerate progress toward a leprosy-free world by 2020, it is fitting to look back on the evolution of progress in treating lepromatous neuropathy and limb deformities. To date, no surgeon has had as great an impact on the understanding and treatment of this disease as Dr Paul Brand. Before Dr Brand's accomplishments, few surgeons participated in the management of the deformed leprous patient. By challenging conventional beliefs, Dr Brand revealed that many of the deformities associated with leprosy were in fact caused by nerve damage and subsequent limb anesthesia. His pioneering work centered on tendon transfers to provide hand and foot mobility to leprous patients, revolutionizing the surgical management of this patient population and restoring functionality to the lives of otherwise stigmatized and functionally handicapped individuals. In the process, he provided us with the surgical principles and techniques that we still apply today. Because of its predilection for the peripheral nervous system, leprosy also provides an excellent opportunity to investigate mechanisms of demyelination and chronic nerve degeneration in nonacute peripheral neuropathies. Processes underlying demyelination of infectious, traumatic, and genetic etiologies overlap and precede the onset of acute neuronal derangement. Glial pathology has been shown to be a common pathological element in leprosy, Charcot-Marie-Tooth type I, multiple sclerosis, and chronic nerve compression injury. The aim of this article is to provide an overview of lepromatous neuropathy with its subsequent deformities as it relates to the pathophysiology, surgical management, and potential therapeutic targets of other modern peripheral neuropathies.

Adaptive shortening of long flexor in patients with claw hand: A short report.

Adaptive shortening of long flexors is the common secondary impairment which can occur in patients with long standing claw hand, particularly in those who do not undergo any supervised physiotherapy programme.¹ The main purpose of this short report is to describe the current physiotherapeutic means which are commonly employed for the management of long flexor tightness. This report further gives an insight into the consequences of the delay in corrective surgery and ultimately describes the dire need for further research on this deformity.² Adaptive shortening of the muscles is a phenomenon which can occur for many reasons, like prolonged immobilisation of the of the body segment, postural imbalance, muscle imbalance, impaired muscle performance due to neuromuscular problems or may be due congenital and acquired deformity.Tightness of the muscles leads to limitation of joint range of motion.³ In leprosy the cause of adaptive shortening of the long flexors is muscle imbalance which occurs due to paralysis of the ulnar nerve at the elbow joint. Adaptive shortening of the muscles is a secondary impairment.4 Secondary impairments have multidimensional effects on the management of the deformity, including delay in surgery for the correction of deformity which in turn is responsible for the unemployment, social stigma and problems in social integration.5 Secondary impairment in some way or other is responsible for the above stated problems, but adaptive shortening of the long flexor (long flexor tightness) is important because sometimes months of therapy are required to attain full muscle length.

Zancolli lasso procedure for correction of paralytic claw hands.

PURPOSE: To evaluate the outcome of 20 claw hands corrected with the Zancolli lasso procedure. METHODS: 20 patients aged 7 to 48 (mean, 28) years with claw hand deformity for a mean of 33.3 months secondary to leprosy (n=17), traumatic ulnar nerve injury (n=2), or tardy ulnar nerve palsy (n=1) underwent the Zancolli lasso procedure, in which the flexor digitorum superficialis (FDS) of the middle finger was divided in to 4 slips (one for each finger) and reattached to itself after passing through the proximal pulley. Deformity, power, movement, and function of the hands were evaluated before and after surgery. RESULTS: The mean duration of surgery was 58.4 (range, 50-107) minutes. The mean duration of follow-up was 18 (range, 12-24) months. Deformity correction was good in 15 patients, fair in 3, and poor in 2. The mean angle improvement was maximum in the little finger, followed by the ring, index, and middle fingers. The transverse metacarpal arch (compared with the normal hand) was increased/reversed in 7 patients and decreased in 13 patients. The grip and pinch strength increased in 13 patients, remained unchanged in 6, and decreased in one. Motion and function of the hand was good in 12 patients, fair in 6, and poor in 2. One patient developed swan neck deformity in the little finger owing to suture tension of the transferred slip, and another had paraesthesia over the index finger. They underwent reoperation using the FDS of the ring finger; no sensory or motor recovery was achieved. CONCLUSION: The Zancolli lasso procedure is easy to perform and does not require extensive postoperative training. It restores synchronous finger flexion and spares other superficialis tendons, thus avoiding swan neck deformity of the fingers.

Correction of the claw hand.

Intrinsic paralysis can be the manifestation of a variety of pathologic entities (stroke, cerebral palsy, Charcot-Marie-Tooth, muscular dystrophy, leprosy, trauma, cervical disease, and compressive and metabolic neuropathies). Patients present with a spectrum of clinical findings dependent on the cause and severity of the disease. The 3 main problems caused by intrinsic weakness of the fingers are clawing with loss of synchronistic finger flexion, inability to abduct/adduct the digits, and weakness of grip. Clawing is defined as hyperextension of the metacarpophalangeal joints and flexion of the interphalangeal joints. This article describes the clinical evaluation and surgical treatment options for claw hand.

Factors affecting functional outcome of surgical correction of claw hand in leprosy.

The objective of this paper is to study the results and the factors that impact the results of claw hand surgery in leprosy. 110 patients who underwent claw hand correction between 2002 and 2006 were followed up and studied. Brand's criteria for objective assessment and a visual analog scale for subjective assessment were used. The factors studied were age, sex, clinical, duration of paralysis, long flexor tightness, degree of contracture and type of surgery. Objective assessment showed Excellent or Good results in 76.6%, Fair in 20% and Poor in 3.4%. Subjective assessment showed that 80.8% were fully satisfied or satisfied. Regression analysis showed that age, degree of contracture,duration of paralysis and long flexor contracture were seen as significant factors impacting results of claw hand correction.

Disability profile in leprosy patients' diagnoses in a rural reference leprosy centre in Ethiopia during 1999-2009.

We assessed the epidemiology of disabilities in leprosy cases treated in a rural hospital over a 10-year period. This is a retrospective data collection using leprosy registers and treatment cards in a rural private mission hospital. Over the 10-year period, 210 patients with leprosy were registered for treatment. One hundred and twenty-eight (61.5%) had disabilities (26.0% grade 1 and 35.6% grade 2): 13.5% ocular disabilities, 44.5% disabilities in hands and 44.7% foot impairment. Patients >19 years had more disabilities (66.7% versus 50.7%) (P = 0.03), especially ocular disabilities (16.7% in >20 versus 6.0% in <20 years) (P = 0.03). This study detected a high prevalence of disabilities.

Multiple grade II deformities in a child: tragic effect of leprosy.

Stigma is almost synonymous with visible deformity and this is what makes leprosy a dreaded disease for the common man. Deformities in children are more distressing, due to the limitation of their physical, academic and social life. An 11-year-old female child was presented with trophic ulceration of right foot and triple nerve paralysis (i.e. total claw hand with wrist drop) of the left hand of 2 years duration. In addition, she had multiple hypopigmented hypoaesthetic patches on the extremities and buttocks. She was diagnosed as a case of borderline tuberculoid leprosy and was started on WHO MB MDT therapy. Leprosy in children is an indicator for disease prevalence in general population and occurrence of deformities points towards the delay in making the diagnosis and the stigma associated with the disease.

Temporoparietal fascial free flap for correction of first web space atrophy.

Fourteen temporoparietal fascial free flaps were used for correction of first web space atrophy from ulnar nerve palsy in 13 patients. Ten sustained ulnar nerve injuries and three suffered from leprosy. The procedures were performed under general anesthesia except one leprosy patient with bilateral ulnar nerve palsy in which local anesthesia and brachial block were employed to harvest bilateral free flaps and recipient site preparations, respectively. The follow-up time varied from 4 to 64 months. The postoperative results were satisfactory and there was no resorption of the free flaps. The consistency of the augmented first web space was soft and compressible like natural feel. The size of the flap was more than enough for augmentation of first web space and donor site morbidity was minimal and accepted by all patients. We conclude that temporoparietal fascial free flap is an ideal autogenous tissue for correction of first web space atrophy.

A randomized clinical trial comparing immediate active motion with immobilization after tendon transfer for claw deformity.

PURPOSE: Immobilization after tendon transfers has been the conventional postoperative management. A recent study indicated beneficial effects of an immediate active motion protocol (IAMP) after tendon transfer for claw deformity correction compared with effects in a historical cohort. In this study, we further tested this hypothesis in a randomized clinical trial comparing the effectiveness of the IAMP with that of conventional immobilization. METHODS: Fifty supple claw hand deformities were randomized postoperatively into 2 equal groups for IAMP and immobilization. Therapy began on the second postoperative day for the IAMP group and on the twenty-second postoperative day for the immobilization group. The primary outcome measures were deformity correction, active range of motion of digits, tendon transfer insertion pullout, and time until discharge from rehabilitation. Secondary outcome measures were swelling, pain, hand strength, and dexterity. Both groups were compared at discharge from rehabilitation and at the last clinical follow-up (at least 1 year postoperatively). RESULTS: Assessments were available for all 50 patients at discharge and for 23 patients in each group at follow-up. The average follow-up was 18 months for the IAMP group and 17 months for the immobilization group. Deformity correction, range of motion, swelling, dexterity, and hand strength were similar for both groups at discharge and a follow-up. There was no evidence of tendon insertion pullout in any patient of either group. Relief of pain was achieved significantly earlier with IAMP. Morbidity was reduced by, on average, 22 days with IAMP. CONCLUSIONS: We found that the immediate active motion protocol is safe and has similar outcomes compared with those of immobilization, with the added advantage of earlier pain relief and quicker restoration of hand function. Immediate motion after tendon transfer can significantly reduce morbidity and speed up the rehabilitation of paralytic limbs, and it may save expense for the patients.

Lepromatous leprosy.

Lepromatous leprosy is a form of chronic granulomatous disease that is caused by infection with Mycobacterium leprae. Early involvement is marked by widespread, ill-defined, erythematous papules and plaques. With early intervention, leprosy is a curable disease; however, if not recognized and treated promptly, permanent sequelae and disability result. We present a patient with long-standing lepromatous leprosy who exhibits many of these sequelae.

Immediate postoperative active mobilization versus immobilization following tendon transfer for claw deformity correction in the hand.

PURPOSE: To test the hypothesis that immediate postoperative active mobilization of tendon transfer following claw correction with flexor digitorum superficialis 4-tail pulley insertion will achieve similar outcomes to immobilization in a cast for 3 weeks. METHODS: In a prospective study, 32 hands with complete ulnar nerve paralysis had flexor digitorum superficialis middle finger 4-tail pulley insertions for 4-digit claw deformity correction and mobilization for tendon transfer rehabilitation on the second day after surgery. Surgical technique was modified to increase the strength of transfer slip insertion. Historical records of 32 mobile claw deformities treated prior to the prospective trial in the same institution with a similar procedure and immobilized in a cast for 3 weeks was used for comparison. Outcomes were assessed by (1) the status of tendon transfer attachment to flexor pulley during immediate mobilization to detect tendon transfer insertion pullout; (2) results of the claw correction in open hand position and intrinsic plus position, and range of digit flexion using identical outcome measures (3) morbidity following surgery; and (4) comparing results of immediate mobilization with immobilization. RESULTS: There was no incidence of transfer insertion pullout during immediate postoperative mobilization. There was no clinically relevant difference in results of claw correction of both groups in open hand and intrinsic plus position. Total active motion of digit flexion was considerably better with immediate mobilization at late result. A reduction of morbidity by 21 days and an earlier return to daily living activities were benefits to the patient with immediate postoperative mobilization of tendon transfer for claw correction. CONCLUSIONS: This study supports the hypothesis. Immediate postoperative active mobilization is safe and has similar outcomes of deformity correction compared to immobilization. Immediate mobilization has the added benefits of reduced morbidity and improved total active range of digit flexion compared to immobilization. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic II.

ECRL--a wrist extensor with multiple uses in deformity correction.

Extensor carpi radialis longus muscle has been used in various types of procedures for corrective hand surgery and is a favored muscle for correction of finger clawing due to ulnar nerve palsy in leprosy because its removal leaves an insignificant motor deficit and gives a linear scar at the donor site. It is usually not paralyzed in leprosy. The muscle, being phasic, is easy to re-educate. The excursion of the muscle is similar to lumbrical muscles which it substitutes. Since the muscle is dorsally located, the transfer does not lose tension due to adaptive wrist flexing habit. Its tendon is usually thick enough, can be split into two and used as graft to elongate the muscle-tendon unit or for ligament reconstruction in cases of trapezio-metacarpal joint arthritis.

[Flexor digitorum superficialis tendon transfer for intrinsic paralysis in leprosy]. / Transfert tendineux palliatif utilisant le Flexor Superficialis Digitorum dans les paralysies intrinsèques d'origine lépreuse.

A follow up study was performed in the rehabilitation centre for patients with leprosy in Hôchiminhville - Vietnam. All patients had claw-hand deformity due to ulnar and median nerve intrinsic paralysis. Thirty-two affected hands (128 long fingers) were included in the study. A Bunnel-Littler tendon transfer procedure was performed using a four-tailed graft of the flexor digitorum superficialis of the third finger. Clinical evaluation included anatomical measurements of interphalangeal and metacarpal joints in complete extension and in the intrinsic position. In the open hand assessment, 48.5% reported good results, 14.8% medium results and 36.7% poor results. With the hand in the intrinsic position, 53.9% achieved good results, while 33.6% achieved medium results and poor in 12.5%. Poor functional outcome is related to a failure of this procedure and seems to be due to extensor tendon laxity, with or without stiffness of the interphalangeal joints. There were many anatomical deformities (27.3%) found at the time of follow up, notably boutonniere (51.4%) and mallet finger deformities (31.4%) The fourth and fifth fingers had the worst results. We have therefore decided to change our protocol for claw-hand correction and use the Bouvier test in deciding on our surgical indications. Preoperative physiotherapy is absolutely necessary to reduce stiffness of the interphalangeal joints.